PROC. NATL. ACAD. SCI. U. S. A. 96/26 (1999) 15137-15142

Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans

Scott M.R., Will R., Ironside J., Nguyen H.-O.B., Tremblay P., DeArmond S.J. , Prusiner S.B.

Inst. for Neurodegenerative Diseases, Department of Neurology, University of California, San Francisco, CA 94143-0518, USA

Abstract
There is growing concern that bovine spongiform encephalopathy (BSE) may have passed from cattle to humans. We report here that transgenic (Tg) mice expressing bovine (Bo) prion protein (PrP) serially propagate BSE prions and that there is no species barrier for transmission from cattle to Tg(BoPrP) mice. These same mice were also highly susceptible to a new variant of Creutzfeldt-Jakob disease (nvCJD) and natural sheep scrapie. The incubation times (≃250 days), neuropathology, and disease-causing PrP lsoforms in Tg(BoPrP)Prnp(0/0) mice inoculated with nvCJD and BSE brain extracts were indistinguishable and differed dramatically from those seen in these mice injected with natural scrapie prions. Our findings provide the most compelling evidence to date that prions from cattle with BSE have infected humans and caused fatal neurodegeneration.

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